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dermatitis herpetiformis; Duhring-Brocq disease

A chronic symmetric, itching vesiculobullous dermatitis. Etiology: 1) ingestion of iodides may precipitate symptoms 2) gluten-sensitive enteropathy (celiac sprue) in nearly all patients (generally asymptomatic) 3) small bowel malabsorption occurs in 10-20% Epidemiology: 1) onset 20-60 years, most commonly 30-40, but may occur in children 2) male:female ratio 2:1 3) 10-11 cases/100,000/year Pathology: (Dermatopathology) 1) microabscesses at the tips of the dermal papillae a) neutrophils b) eosinophils c) results in subepidermal separation [5] 2) fibrin accumulation & necrosis 3) dermal infiltration of neutrophils & eosinophils 4) subepidermal vesicle 5) IgA deposits in affected & normal-appearing skin a) granular in tips of papillae 1] correlates with small bowel disease 2] found in majority of patients 3] complement also deposited b) linear, bandlike along dermal-epidermal junction 1] other bullous diseases may have IgG in band-like pattern beneath the epidermal basement membrane 2] pattern not associated with small bowel disease 3] pattern in minority of patients c) IgA associated with microfibrils d) IgA & complement mediate cascade of events leading to tissue injury e) IgA antibodies to transglutaminase (TG3) [6] * histopathology images [11] Genetics: - IL31 is up-regulated in lesional biopsies of patients with allergic contact dermatitis Clinical manifestations: 1) intense, episodic pruritus a) rarely, pruritus may not occur b) burning or stinging sensation may occur instead c) pruritus may precede lesions by 8-12 hours 2) erythematous papules, vesicles, bullae (occasionally) 3) urticaria-like wheal 4) scratching results in excoriations with crusting; may obscure visualization of vesicles or bullae [5] 5) post-inflammatory hyperpigmentation at sites of healed lesions 6) lesions arranged in groups (crops), symmetrically distributed 7) sites of predilection: a) extensor areas 1] elbows 2] knees b) buttocks c) scapular area d) sacral area e) scalp & hairline f) face 8) does not involve oral mucosa [5] * images [11,12] Laboratory: 1) eosinophilia 2) HLA typing: association with HLA-B8, HLA-DR3 & HLA-DQ2 3) serology: a) circulating antibodies to basement membrane generally not detectable b) IgG & IgA anti-reticulin antibodies may be present c) antimicrosomal antibodies may be present d) antinuclear antibodies may be present e) circulating immune complexes in 20-100% f) IgA anti-endomysial antibodies 1] bind to intermyofibril substance of smooth muscle 2] present in the majority of patients 3] correlate with severity of intestinal disease g) IgA antibodies to transglutaminase (TG2, TG3) [6] 4) malabsorption studies a) steatorrhea (20-30%) b) abnormal D-xylose absorption (10-73%) 5) anemia secondary to iron or folate deficiency a) complete blood count (CBC) b) iron sudies 1] serum iron, TIBC, serum ferritin 2] reticulocyte count c) serum folate 6) erythrocyte glucose-6-phosphate dehydrogenase (erythrocyte G6PD) level prior to treatment with sulfones 7) skin biopsy a) best from early erythematous papule b) immunofluorescence of normal-appearing skin 8) see ARUP consult [7] Special laboratory: - upper GI endoscopy a) blunting & flattening of the villi (80-90%) b) small bowel biopsy Differential diagnosis: 1) allergic contact dermatitis 2) atopic dermatitis 3) scabies 4) neurotic excoriations 5) papular urticaria 6) bullous pemphigoid 7) herpes gestationis Complications: - increased risk of lymphoma [9] Management: 1) dapsone 100-200 mg QD with taper to 25-50 mg QD a) measure serum G6PD prior to treatment with dapsone b) relieves pruritus & clears skin lesions in 24-48 hours by blocking IgA-mediated neutrophil chemotaxis c) follow CBC weekly for 1st month, then every 6-8 weeks 2) sulfapyridine 1.0-1.5 g QD with fluids, if dapsone contraindicated or not tolerated 3) gluten-free diet (1st line) [5] a) may completely suppress symptoms or allow reduction of dapsone or sulfapyridine b) response to gluten-free diet is slow 4) prognosis a) prolonged course, over years b) 1/3 of patients eventually have spontaneous remission c) relapses are common

Interactions

disease interactions

Related

atopic dermatitis (atopic eczema) bullous pemphigoid; parapemphigus celiac sprue (gluten-sensitive enteropathy) contact dermatitis (exogenous eczema) scabies urticaria (hives)

General

vesiculobullous dermatitis

References

  1. Stedman's Medical Dictionary 26th ed, Williams & Wilkins, Baltimore, 1995
  2. DeGowin & DeGowin's Diagnostic Examination, 6th edition, RL DeGowin (ed), McGraw Hill, NY 1994, pg 878
  3. Color Atlas and Synopsis of Clinical Dermatology, Common and Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 325-27
  4. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 168
  5. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2018, 2021.
  6. Hull CM et al. Elevation of IgA anti-epidermal transglutaminase antibodies in dermatitis herpetiformis. Br J Dermatol 2008 Jul; 159:120. PMID: 18503599
  7. ARUP Consult: Dermatitis Herpetiformis The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/dermatitis-herpetiformis
  8. Zone JJ. Skin manifestations of celiac disease. Gastroenterology. 2005 Apr;128(4 Suppl 1):S87-91. PMID: 15825132
  9. Hervonen K, Vornanen M, Kautiainen H, Collin P, Reunala T Lymphoma in patients with dermatitis herpetiformis and their first-degree relatives. Br J Dermatol. 2005 Jan;152(1):82-6. PMID: 15656805
  10. Karpati S Dermatitis herpetiformis. Clin Dermatol. 2012 Jan-Feb;30(1):56-9. PMID: 22137227
  11. Miller JL, Elston DM (images) Medscape: Dermatitis Herpetiformis http://emedicine.medscape.com/article/1062640-overview
  12. DermNet NZ. Dermatitis herpetiformis (images) http://dermnetnz.org/immune/dermatitis-herpetiformis.html
  13. Jakes AD, Bradley S, Donlevy L. Dermatitis herpetiformis. BMJ. 2014 Apr 16;348:g2557. PMID: 24740905
  14. Reunala T, Hervonen K, Salmi T Dermatitis Herpetiformis: An Update on Diagnosis and Management Am J Clin Dermatol. 2021 May;22(3):329-338 PMID: 33432477 PMCID: PMC8068693 Free PMC article